Prognosis of Ocular Myasthenia Gravis in an Argentinian Population

Background: We investigated the prognosis of patients with myasthenia gravis (MG) initially presenting with only ocular symptoms in an Argentinian population. Summary: We performed a retrospective analysis of 61 patients with MG with pure ocular involvement at onset. Generalized MG (gMG) developed in 73.7% of patients, while the rest only exhibited ocular symptoms throughout the course of the disease (ocular MG [oMG]). The AChR antibody (AChR-ab) was found in 81.4% of the gMG and 50% of the oMG cases. AChR-ab titers were also significantly higher in the gMG than in the oMG group. Patients with oMG more frequently required treatment only with acetylcholinesterase inhibitors and showed more remissions. Key Messages: Our study showed high generalization in patients with MG debuting with ocular symptoms. Patients with AChR seropositivity and higher AChR-ab titers had an increased risk for gMG, while patients with oMG showed lower corticosteroid requirements and a higher remission rate.

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Thymectomy in ocular myasthenia gravis before generalization results in a higher remission rate

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezz275 ◽

2019 ◽

Cited By ~ 2

Author(s):

Feng Li ◽

Zhongmin Li ◽

Yanli Chen ◽

Gero Bauer ◽

Deniz Uluk ◽

...

Keyword(s):

Myasthenia Gravis ◽

Remission Rate ◽

Post Intervention ◽

Data Set ◽

Ocular Myasthenia ◽

Ocular Symptoms ◽

Ocular Myasthenia Gravis ◽

Before And After ◽

Robotic Thymectomy ◽

Lost To Follow Up

Abstract OBJECTIVES This study aimed to compare the outcomes of patients with ocular myasthenia gravis (OMG) who underwent thymectomy before generalization with the outcomes of those who underwent thymectomy after generalization. METHODS We retrospectively reviewed patients who underwent robotic thymectomy for myasthenia gravis between January 2003 and February 2018. Patients who presented with purely ocular symptoms at myasthenia gravis onset were eligible for inclusion. Exclusion criteria were patients who were lost to follow-up and patients who underwent re-thymectomy. Patients with OMG who developed generalization before thymectomy were categorized into gOMG group and those who did not were categorized into OMG group. The primary outcome was complete stable remission according to the Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS). RESULTS One hundred and sixty-five (66 males and 99 females) out of 596 patients with myasthenia gravis were eligible for inclusion. Of these, there were 73 and 92 patients undergoing thymectomy before and after the generalization of OMG, respectively. After propensity score matching, a data set of 130 patients (65 per group) was formed and evaluating results showed no statistical differences between the 2 groups. The estimated cumulative probabilities of complete stable remission at 5 years were 49.5% [95% confidence interval (CI) 0.345–0.611] in the OMG group and 33.4% (95% CI 0.176–0.462) in the gOMG group (P = 0.0053). Similar results were also found in patients with non-thymomatous subgroup [55 patients per group, OMG vs gOMG, 53.5% (95% CI 0.370–0.656) vs 28.9% (95% CI 0.131–0.419), P = 0.0041]. CONCLUSIONS Thymectomy in OMG before generalization might result in a higher rate of complete stable remission than thymectomy after generalization.

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THUR 231 Developing a new rating scale for ocular myasthenia gravis

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-abn.116 ◽

2018 ◽

Vol 89 (10) ◽

pp. A33.3-A33

Author(s):

Sui H Wong ◽

Eduardo Miranda ◽

Helena Lee ◽

Eric Eggenberger ◽

Wayne Cornblath ◽

...

Keyword(s):

Myasthenia Gravis ◽

Rating Scales ◽

Rating Scale ◽

Observational Cohort Study ◽

Ocular Myasthenia ◽

Ocular Symptoms ◽

Composite Scale ◽

Ocular Myasthenia Gravis ◽

Observational Cohort ◽

Future Work

Ocular Myasthenia Gravis (OMG) causes ptosis and diplopia, which can be disabling. For this study, OMG is defined as MG patients who have ocular symptoms only and no generalised involvement. A robust way of assessing the severity of OMG symptoms is important for research, to assess treatment and outcome. The rating scales recommended for MG research have a predominant focus on generalised disease, and are insufficiently sensitive for OMG due to the limited number of ocular questions. This study aims to create a new rating scale for OMG that is sensitive, reliable and clinically usable. We present our proposal of such a rating scale, which incorporates physician- and patient-rated components. We report the preliminary results of this pilot observational cohort study, in 60 patients with OMG. We compare the results of this with the MG composite scale.Future work is planned to validate this rating scale and to develop this alongside the MG Impairment Index (MGII). For future phases of this study we plan to assess the usability of this rating scale by Neurologists without specialist Neuro-ophthalmology.We invite feedback from Neurologists at the ABN.

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Adult Ocular Myasthenia Gravis Conversion: A Single-Center Retrospective Analysis in China

European Neurology ◽

10.1159/000507853 ◽

2020 ◽

Vol 83 (2) ◽

pp. 182-188

Author(s):

Xuelin Feng ◽

Xiao Huan ◽

Chong Yan ◽

Jie Song ◽

Jun Lu ◽

...

Keyword(s):

Risk Factors ◽

Survival Analysis ◽

Myasthenia Gravis ◽

Conversion Rate ◽

Cox Regression ◽

Ocular Involvement ◽

Onset Age ◽

Bilateral Ptosis ◽

Ocular Myasthenia ◽

Ocular Myasthenia Gravis

Introduction: The conversion rate from ocular myasthenia gravis (OMG) to generalized myasthenia gravis (GMG) was reported to be much lower in Asian population since most OMG patients are juvenile onset. However, the exact conversion rate for adult-onset OMG to GMG is still unknown. Objective: We aimed to delineate the conversion rate and risk factors for adult patients with ocular onset to GMG. Methods: Adult myasthenia gravis (MG) patients with ocular onset (age > 18 years) were retrospectively reviewed. Patients with confined ocular involvement lasting more than 2 years (pure OMG group) and those who converted into GMG (converted OMG group) were enrolled for subsequent analysis. We then analyzed 5 clinical variables, including onset age, sex, onset symptoms, anti-acetylcholine receptor antibody (AChR Ab), and thymus CT. Survival analysis was applied to all enrolled patients to explore risk factors associated with conversion. Results: In a total number of 249 ocular-onset MG patients initially enrolled, we excluded 122 patients with OMG lasting less than 2 years. The remaining 127 patients were enrolled, including 106 converted OMG and 21 pure OMG patients. Converted OMG patients had an older onset age (threshold: 43 years) and higher anti-AChR Ab titer (threshold: 6.13 nmol/L). The estimated conversion rate was 70.64%. Moreover, 67% of conversion occurred within 2 years after onset. Cox regression of survival analysis revealed that higher anti-AChR Ab titer and bilateral ptosis were associated with a higher conversion rate. Conclusions: The conversion of adult OMG was associated with anti-AChR Ab titer, onset age, and bilateral ptosis. The estimated conversion rate of Chinese adult OMG patients was 70%.

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Pure Ocular Myasthenia gravis

Neurology International Open ◽

10.1055/s-0043-122245 ◽

2018 ◽

Vol 02 (01) ◽

pp. E51-E55

Author(s):

Lukas Kirzinger ◽

Sophie Schötz ◽

Berthold Schalke

Keyword(s):

Neuromuscular Junction ◽

Myasthenia Gravis ◽

Diagnostic Procedure ◽

Cholinesterase Inhibitors ◽

Separate Entity ◽

Ocular Myasthenia ◽

Randomized Controlled ◽

Ocular Symptoms ◽

Randomized Controlled Studies ◽

Ocular Myasthenia Gravis

AbstractPure ocular myasthenia was always described as a separate entity in historical publications. More than 50% of patients develop generalized symptoms in the course of the disease. Therefore pure ocular symptoms can be the beginning of generalized myasthenia, nevertheless there is a small group of patients who suffer lifelong purely ocular symptoms. The basis, therefore, seems to be special immunological, biochemical and pathophysiological mechanisms leading to specific local changes of the neuromuscular junction. The diagnostic procedure is hampered as antibodies against acethylcholine receptors and MuSK can be demonstrated in less than 50% of these patients. Therapy of pure ocular myasthenia does not differ fundamentally from other subgroups of myasthenia gravis and is implemented with cholinesterase inhibitors, steroids and common immunosuppressants. Thymomectomy is not recommended in general, but randomized controlled studies are lacking.

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Mitral Valve Replacement with Thymectomy in a Patient with Ocular Myasthenia Gravis: Case Report

The Heart Surgery Forum ◽

10.1532/hsf.2627 ◽

2019 ◽

Vol 22 (5) ◽

pp. E340-E342

Author(s):

Ovidiu Stiru ◽

Mihai Stefan ◽

Roxana Carmen Geana ◽

Diana Sorostinean ◽

Răzvan Radu ◽

...

Keyword(s):

Mitral Valve ◽

Myasthenia Gravis ◽

Valve Replacement ◽

Mitral Valve Replacement ◽

Mitral Valve Stenosis ◽

Management Problem ◽

Ocular Myasthenia ◽

Anaesthetic Agents ◽

Ocular Symptoms ◽

Ocular Myasthenia Gravis

Patients diagnosed with ocular myasthenia gravis (MG) and mitral valve disease represent a significant perioperative management problem, especially for the anaesthesiologist, due to complex inter-actions between the disease, drugs to treat the disease, and anaesthetic agents, such as neuromuscu-lar blocking agents (NMBAs). This paper describes the successful management of a 31-year-old female with mitral valve stenosis and ocular MG who was diagnosed with MG 4 years prior to the indication for cardiac surgery. Preoperatively, the patient was under treatment with Pyridostigmine and Prednisone. Mitral valve replacement and full thymectomy were performed, under general anaesthesia, using Fentanyl, Sevoflurane and low doses of non-depolarising NMBAs. The postoperative course was uneventful, the patient was extubated at 6 hours postoperatively, in-tensive care unit stay was 48 hours, and the patient was discharged after 6 days without any compli-cations. After 3 months, at the follow-up examination, the patient’s ocular symptoms (eyelid ptosis) disappeared.

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Abstract #1154: Ocular Myasthenia Gravis in the Setting of Graves’ Disease

Endocrine Practice ◽

10.1016/s1530-891x(20)44799-8 ◽

2016 ◽

Vol 22 ◽

pp. 277-278

Author(s):

Lima Lawrence ◽

Aleida Rodriguez ◽

Tahira Yasmeen ◽

Erin Drever

Keyword(s):

Myasthenia Gravis ◽

Graves Disease ◽

Ocular Myasthenia ◽

Ocular Myasthenia Gravis

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The diagnosis and management of ocular myasthenia gravis

East European Journal of Parkinson`s Disease and Movement Disorders ◽

10.33444/2414-0007.5.1-2.3-8 ◽

2019 ◽

Vol 5 (1-2) ◽

pp. 3-8

Author(s):

Gordon Plant

Keyword(s):

Myasthenia Gravis ◽

Severe Form ◽

Differential Diagnostics ◽

Diagnosis And Management ◽

Ocular Myasthenia ◽

Clinical Presentations ◽

Ocular Myasthenia Gravis

Ocular myasthenia is not the most severe form of myasthenia but it might cause certain difficulties to diagnose. In this article, we shall discuss the clinical presentations of ocular myasthenia, its differential diagnostics, examinations and management. Keywords: ocular myasthenia gravis, diagnosis, examination, management.

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